Matching the pitch perception of the cochlear implanted ear with the contralateral ear in patients with single-sided deafness: a novel approach.

PURPOSE: Single-sided deaf patients following cochlear implantation often compare the sound quality of their implanted ear with normal hearing. The interaural differences can result in dissatisfaction with speech comprehension and reduced time of usage of the speech processor; hence, prolonging auditory adaptation time. The proposed calibration method presented in this study demonstrates how the frequency distribution of the cochlear implant can be set to adequately approximate the pitch perception of the contralateral normal hearing ear towards improving speech intelligibility in a noisy environment. METHODS: In 12 postlingual single-sided deaf patients, subjective interaural pitch-matching was carried out to determine new central frequencies for the reallocation of the frequency bands of their speech processor (CP910, CP950 or CP1000, Cochlear, Australia). The patients were asked to compare the pitch of the tones presented to their normal hearing ear to the pitch of individual channels of their cochlear implant (CI522 or CI622, Cochlear, Australia). A third-degree polynomial curve was fit to the acquired matching frequencies to create the new frequency allocation table. Audiological measurements (free-field aided thresholds, speech reception thresholds, and monosyllabic word recognition score) in noise, together with a Speech, Spatial and Qualities of Hearing Scale (SSQ12) questionnaire (short version of the original SSQ) results were evaluated prior to the pitch-matching procedure, and again, 2 weeks later. RESULTS: The free-field aided thresholds of the patients showed no greater shift than ± 5 dB following the procedure; however, their monosyllabic word recognition score in noise improved significantly (mean - 9.58%, SD 4.98%, matched pairs t test comparison: p < 0.001). The results of the SSQ12 questionnaire also showed significant improvement in speech intelligibility, sound localization, and sound quality (mean 0.96 points, SD 0.45 points, matched pairs t test comparison: p < 0.001). CONCLUSIONS: Matching the pitch perception of the implanted cochlea with the sensation of the normal hearing contralateral ear, resulted in significant changes in the quality of hearing in patients with single-sided deafness. It is plausible the procedure can usher positive results in bimodal patients or following sequential bilateral cochlear implantation.

Genetic Etiology of Nonsyndromic Hearing Loss in Hungarian Patients.

Hearing loss is the most prevalent sensory disorder worldwide. The majority of congenital nonsyndromic hearing loss (NSHL) cases are caused by hereditary factors. Previously, the majority of NSHL studies focused on the GJB2 gene; however, with the availability of next-generation sequencing (NGS) methods, the number of novel variants associated with NSHL has increased. The purpose of this study was to design effective genetic screening for a Hungarian population based on a pilot study with 139 NSHL patients. A stepwise, comprehensive genetic approach was developed, including bidirectional capillary sequencing, multiplex ligation-dependent probe amplification (MLPA), and an NGS panel of 108 hearing loss genes. With our results, a genetic diagnosis was possible for 92 patients. Sanger sequencing and MLPA identified the genetic background of 50% of these diagnosed cases, and the NGS panel identified another 16%. The vast majority (92%) of the diagnosed cases showed autosomal recessive inheritance and 76% were attributed to GJB2. The implementation of this stepwise analysis markedly increased our diagnostic yield and proved to be cost-effective as well.

Historical overview and surgical applications of the Vibrant Soundbridge active middle ear implant / A Vibrant Soundbridge aktív középfül-implantátum történeti áttekintése és sebészi alkalmazásának lehetoségei.

Összefoglaló. A Vibrant Soundbridge aktív középfül-implantátum vezetéses, sensorineuralis és kevert típusú halláscsökkenés esetén is megoldást jelenthet a beteg hallásrehabilitációja során. Sensorineuralis halláscsökkenés esetén akkor indikálható, ha a légvezetéses hallásküszöb nem haladja meg a 80-85 dBHL-t a 3-4 kHz frekvenciákon, vezetéses, illetve kevert típusú halláscsökkenés esetén pedig akkor, ha a csontvezetéses hallásküszöb nem haladja meg a 45-65 dBHL-t a 0,5-4 kHz közti frekvenciatartományban. Az implantátum beültetését eleinte tisztán sensorineuralis halláscsökkenés esetén végezték, és csupán egyféleképpen történhetett: a rezgéskelto rendszer oldalán lévo rögzítocsipeszt rá kellett applikálni az incus hosszú nyújtványára. Azokra az esetekre, amikor a rezgéskelto rögzítése nem kivitelezheto, különbözo rögzítoelemeket (coupler) fejlesztettek ki. Az incusra való rögzítés hosszúnyújtvány-couplerrel, illetve rövidnyújtvány-couplerrel lehetséges. Vezetéses és kevert típusú halláscsökkenés esetén a kerek ablak membránjához is illesztheto a rendszer, elorehaladott otosclerosis esetén pedig a Soundbridge-implantáció stapedotomiával kiegészített változata ("power stapes") hozhat kielégíto halláseredményt. Ezek a technikák meglehetosen megnövelték a sebész szabadságát, így széles körben alkalmazott, megbízható megoldássá váltak. A Pécsi Tudományegyetem Fül-Orr-Gégészeti és Fej-Nyaksebészeti Klinikáján az elmúlt évtizedben számos incusvibroplastica történt, de kerekablak-vibroplasticára is több alkalommal sor került. A jelen összefoglaló tanulmányban a Vibrant Soundbridge implantátum technikai fejlodésének történeti áttekintésén felül az alkalmazható mutéti megoldásokat mutatjuk be. Orv Hetil. 2021; 162(40): 1619-1626. Summary. The Vibrant Soundbridge active middle ear implant can provide a reliable solution for hearing rehabilitation of patients with conductive, sensorineural or mixed hearing loss. For sensorineural hearing loss, the air conduction threshold of the patient should not be more increased than 80-85 dBHL at the range of 3-4 kHz, and for conductive and mixed hearing loss, the bone conduction threshold should not be more increased than 45-65 dBHL between 0.5 and 4 kHz. The standard surgical procedure was originally designed for purely sensorineural hearing loss, and the fixation clip of the vibrating transducer needed to be crimped onto the long process of the incus. In many cases, it is impossible to crimp the vibrator onto the incus. In order to solve such circumstances, fixation clips (couplers) have been developed. There are two options to crimp the device on the incus: applying a long process coupler or a short process coupler. For conductive or mixed hearing loss, a round window soft coupler has been introduced. In advanced otosclerotic cases, a special combined technique of Soundbridge implantation with simultaneous stapedotomy can result in sufficient hearing rehabilitation. These techniques significantly broadened the scale of possibilities for the implantation, therefore, it became a widely utilized, reliable procedure. At the Department of Otorhino-laryngology, Clinical Center, University of Pécs, in addition to a noteworthy amount of incus vibroplasty, several cases of round window vibroplasty have also been performed. The aim of the present study is to summarize the history of development of the Vibrant Soundbridge and to present an overview of the applicable surgical techniques. Orv Hetil. 2021; 162(40): 1619-1626.

Is the bispectral index monitoring protective against postoperative cognitive decline? A systematic review with meta-analysis.

BACKGROUND: Several studies have suggested that monitoring the depth of anaesthesia might prevent the development of postoperative cognitive decline. We aimed to conduct a meta-analysis to investigate the effects of bispectral index (BIS) monitoring in anaesthesia. METHODS: We searched in six major electronic databases. Trials were included if they discussed anaesthesia with and without BIS monitoring or low (<50) and high (≥50) BIS levels and which measured the risk of postoperative delirium (POD) and/or postoperative cognitive dysfunction (POCD). RESULTS: We included fourteen studies in the systematic review, eight of which were eligible for meta-analysis. BIS proved to be protective against POD at 1 day postoperatively in a cohort of 2138 patients (16.1% vs. 22.8% for BIS vs. no BIS groups, respectively; relative risk [RR] 0.71; 95% confidence interval [CI] 0.59 to 0.85, without significant between-study heterogeneity I2 = 0.0%, P = 0.590). The use of BIS was neutral for POCD at 1 week but protective for POCD at 12 weeks (15.8% vs. 18.8% for BIS vs. no BIS groups, respectively; RR = 0.84, CI: 0.66 to 1.08), without significant between-study heterogeneity (I2 = 25.8%, P = 0.260). The neutral association at 1 week proved to be underpowered with trial sequential analysis. In the comparison of low BIS versus high BIS, the incidence of POD at 1 day was similar in the groups. CONCLUSION: Our findings suggest a protective effect of BIS compared to not using BIS regarding the incidence of POD at 1 day and POCD at 12 weeks. However, limitations of the evidence warrant further investigation to identify those groups of patients by age, comorbid conditions and other individual variables who would benefit the most from the use of BIS monitoring.

Endoscopic type I tympanoplasty is as effective as microscopic type I tympanoplasty but less invasive-A meta-analysis.

BACKGROUND: Endoscopic type I tympanoplasty was originally introduced in the 1990s, and the extensive spread of this practice can be easily observed. The conventional technique performed involves the repair of a tympanic membrane perforation, and is defined as microscopic type I tympanoplasty. OBJECTIVE OF REVIEW: The aim of this study was the comparison of postoperative outcomes of both the endoscopic and the microscopic type I tympanoplasty. TYPE OF REVIEW: We conducted a meta-analysis in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) guidelines. SEARCH STRATEGY: A systematic literature search was performed in the databases of PubMed, Embase, Cochrane Library, Clarivate Analytics-Web of Science, ClinicalTrials.gov, World Health Organization Library, and Scopus by inserting, 'myringoplasty OR (tympanoplasty AND perforation)' into the search query. We applied only a 'human' filter. We excluded non-English studies. Additional records were identified by checking the references of relevant studies. EVALUATION METHOD: Comparative studies were included in our analysis. We calculated the pooled odds ratio (OR) with 95% confidence interval (CI) for dichotomous outcomes and weighted mean difference (WMD) with a 95% CI for continuous outcomes. Additionally, we assessed the risk of bias and estimated the quality of evidence for each outcome. RESULTS: Our systematic search yielded 16 studies (involving 1179 interventions), eligible for analysis. The pooled graft uptake rate (OR: 1.21, CI: 0.82-1.77; I2  = 0.0%), the postoperative hearing results (WMD = -1.13; 95% CI: -2.72-0.45; I2  = 78.1%) and the operation time (WMD = -21.11; 95% CI: -42.60-0.38; I2  = 99.3%), were all comparable amongst the two techniques. In contrast, the endoscopic type I tympanoplasty outperforms when regarding the pooled canaloplasty rate (OR = 7.96; 95% CI: 4.30-14.76; I2  = 0.0%, P = 1.000) and features an increase in desirable cosmetic results (OR = 19.29; 95% CI: 11.37-32.73; I2  = 0.0%, P = 0.839), when compared with the microscopic approach. CONCLUSIONS: Based on our meta-analysis, the surgical outcomes of endoscopic type I tympanoplasty in terms of graft uptake rate, postoperative hearing results and operation time were comparable to the microscopic type I tympanoplasty. In regards to cosmetics, an increase in desirable results was achieved in the endoscopic group, particularly the incidence of canaloplasty which proved to be significantly lower.

[Analysis of prognostic factors influencing the effectiveness of treatment in sudden sensorineural hearing loss]. / A hirtelen halláscsökkenés javulásában szerepet játszó prognosztikai faktorok vizsgálata.

Sudden sensorineural hearing loss is defined as a greater than 30 dB deterioration in at least 3 consecutive frequencies occurring within 72 hours. The disease is mostly idiopathic and needs an urgent treatment. Between 01. 01. 2015 and 31. 12. 2016, 149 patients with sudden hearing loss were admitted to the Department of Otorhinolaryngology, University of Pécs. The epidemiological data, the prognostic factors and the outcome of the treatment were retrospectively analyzed. Isolated high frequency (p = 0.012) and less severe hearing loss (p<0.001), older age (p = 0.005), comorbodities like cardiovascular disease (p = 0.009) and diabetes (p = 0.029) were found as negative prognostic factors. Time of introduction of the treatment, gender, and side of the affected ear did not influence the outcome. Our finding was mainly congruent to the literature. Orv Hetil. 2019; 160(18): 687-693.

Sedation of children for auditory brainstem response using ketamine-midazolam-atropine combination - a retrospective analysis.

Authors investigated sedation quality in children for auditory brainstem response testing. Two-hundred and seventy-six sedation procedures were retrospectively analyzed using recorded data focusing on efficacy of sedation and complications. Intramuscular ketamine-midazolam-atropine combination was administered on sedation preceded by narcotic suppository as pre-medication. On using the combination vital parameters remained within normal range, the complication rate was minimal. Pulse rate, arterial blood pressure and pulse oxymetry readings were stable, hypoventilation developed in 4, apnoea in none of the cases, post-sedation agitation occurred in 3 and nausea and/or vomiting in 2 cases. Repeated administration of narcotic agent was necessary in a single case only. Our practice is suitable for the sedation assisting hearing examinations in children. It has no influence on the auditory brainstem testing, the conditions necessary for the test can be met entirely with minimal side-effects. Our practice provides a more lasting sedation time in children during the examination hence there is no need for the repetition of the narcotics.

PACAP ameliorates oxidative stress in the chicken inner ear: an in vitro study.

Pituitary adenylate cyclase activating polypeptide (PACAP) is a pleiotropic and multifunctional neuropeptide. Numerous studies prove that PACAP has neuroprotective effects in diverse neuronal systems in vitro and in vivo. The involvement of PACAP in visual and olfactory sensory processing has also been documented, but little is known about its effects in the auditory system. The presence of PACAP and its receptor, the specific PAC1 receptor, has been shown in the cochlea and in brain structures involved in auditory pathways. The aim of the present study was to investigate whether PACAP is protective in cochlear oxidative stress-induced cell death, which is known to play a role in several ototoxic insults. Chicken cochlear cells were exposed to 1mM H(2)O(2), which resulted in a marked reduction of cell viability and a parallel increase of apoptotic and necrotic cells assessed by MTT test, annexin V/propidium iodide flow cytometry and JC-1 apoptosis assay. Co-incubation with 100nM PACAP increased cell viability and reduced the percentage of apoptotic cells. Furthermore, oxidative stress increased the activation of caspase-3, while simultaneous PACAP treatment reduced it. In summary, our present results demonstrate that PACAP effectively protects cochlear cells against oxidative stress-induced apoptotic cell death.

[Susac syndrome: neurologic, psychiatric, ophthalmologic otorhinolaryngologic and neuroradiologic features of a rare autoimmune disease]. / Susac-szindróma: egy ritka autoimmun kórkép neurológiai, pszichiátriai, szemészeti, fül-orr-gégészeti és neuroradiológiai vonatkozásai.

UNLABELLED: Encephalopathy, recurrent occlusion of retinal arteries and hearing loss comprise the clinical picture of Susac's syndrome. The correct diagnosis is frequently missed because of incomplete clinical signs or negligence of previous symptoms. Early diagnosis and treatment can halt the progression and prevent permanent disability. METHODS: Here, we describe a Hungarian case and review the clinical characteristics, diagnostic procedures and current concepts of therapy. RESULTS: A 30-year-old female was admitted to our neurology department because of change in her personality, apathy, and difficulty in concentration. Brain MRI indicated multiple hyperintense T2-weighted lesions including cerebellum and corpus callosum. Protein content of the CSF was markedly elevated. The recurrent bilateral loss of vision and hearing along with migraine in her previous 2,5-year-long medical history suggested Susac's syndrome. Fundoscopy and fluorescein angiography indicated multiple occlusions of the retinal arteries, audiography revealed bilateral hearing loss. Systemic autoimmune and connective tissue diseases and thrombophilia were excluded. The markedly elevated protein in the cerebrospinal fluid supported Susac's syndrome. Chronic treatment with methylprednisolone resulted in remission of clinical signs. DISCUSSION: Consideration of multiple clinical signs is an important key to the diagnosis of rare clinical entities like Susac's syndrome.